Long-term results of surgical treatment of dysphagia secondary to cervical diffuse idiopathic skeletal hyperostosis

Background contextLarge, prominent osteophytes along the anterior aspect of the cervical spine have been reported as a cause of dysphagia. Improvement of swallowing after surgical resection has been reported in a few case reports with short-term follow-up. The current report describes outcomes of a series of five patients with surgical treatment for this rare disorder, with a long-term follow-up.PurposeTo study the clinical and radiographic outcomes of a case series of patients surgically treated for dysphagia secondary to cervical diffuse idiopathic skeletal hyperostosis (DISH).Study designRetrospective review of a case series.Patient sampleFive cases from a University Hospital.Outcome measuresClinical and imagenological follow-up.MethodsThe records of five patients with dysphagia who had undergone anterior surgical resection of prominent osteophytes secondary to DISH were reviewed. Extrinsic esophageal compression secondary to anterior cervical osteophytes was radiographically confirmed via preoperative barium esophagogram swallowing study. All patients underwent anterior cervical osteophytes resection without fusion. Postoperatively, patients were followed-up clinically and radiographically with routine lateral cervical radiographs.ResultsPreoperative esophagogram showed that the esophageal obstruction was present at one level in three cases and two levels in two cases. The C3–C4 level was involved in three cases, C4–C5 in three cases, and C5–C6 in one case. There were no postoperative complications, including recurrent laryngeal nerve palsy, wound infection, or hematomas. All patients had resolution of dyphagia soon after surgery (within 2 weeks). Postoperative radiographs demonstrated complete removal of osteophytes. At final follow-up, ranging from 1 to 9 years (average 59.8 months, median 53 months), no patients reported recurrence of dysphagia. Final radiographic examination demonstrated minimal regrowth of the osteophytes.ConclusionsAlthough rarely indicated, surgical resection of anterior cervical osteophytes from DISH causing dyphagia produces good clinical and radiographical outcomes. After thorough evaluation to rule out other intrinsic or extrinsic causes of swallowing difficulty, surgical treatment of this uncommon condition might be considered.     

Cognitive and neuroradiological findings in myotonic dystrophy.

Cognitive functions were investigated in 37 patients with myotonic dystrophy (MD) and correlated with clinical and neuroradiological variables. The whole cognitive performance was at a low-average level; in about 1/3 of the subjects, in fact, the scores at the neuropsychological tests were below the normal range. There was a consistent trend for patients with inheritance on maternal side to perform worse on Wechsler verbal score and to present cerebral atrophy. In 7 out of 12 subjects focal white matter lesions were found at nuclear magnetic resonance. The significance of these findings and its relation to cognitive performance are discussed.     

Adrenal function under long-term raloxifene administration.

The aim of the present study was to evaluate the effect of long-term (12 months) administration of raloxifene hydrochloride (60 mg/day) on the steroid production of the adrenal cortex and on the hypothalamic-pituitary-adrenal axis in postmenopausal women. We performed a basal evaluation, a corticotropin releasing factor (CRF) (100 microg i.v. bolus) test and a dexamethasone (DXM) (0.25 mg) suppression-adrenocorticotropic hormone (ACTH) (10 microg i.v. bolus) stimulation test in 11 postmenopausal women, before and after 3, 6 and 12 months of raloxifene treatment. Raloxifene administration significantly modified circulating levels of adrenal steroids, decreasing cortisol (-24%), dehydroepiandrosterone (DHEA) (-36%), and its sulfate (DHEAS) (-41%), and androstenedione (-29%), and increasing circulating allopregnanolone (+39%) levels. Progesterone and 17OH-progesterone levels remained unmodified, while estradiol and estrone levels showed a significant decrease (-51% for estradiol and -61% for estrone). We also observed an increase in circulating ACTH (+58%) and beta-endorphin (+120%). No modifications in the hormonal responses to CRF were observed during the treatment. DXM significantly suppressed circulating steroids at any time with a lower suppression of cortisol from the third month and a higher suppression of DHEA at 12 months. ACTH administration was associated with a significantly blunted cortisol response from the sixth month and a significantly increased response of allopregnanolone from the third month. The present data exclude a raloxifene effect on pituitary sensitivity to CRF and demonstrate a reduced adrenal sensitivity to ACTH, sustained by the opposite changes in basal cortisol and Delta5 androgens, which were reduced, and in ACTH and beta-endorphin, which were increased, as well by the reduced response of cortisol to the direct ACTH stimulus. The reduction of circulating cortisol levels and cortisol response to the ACTH challenge suggests that raloxifene protects against the neurotoxic effects of endogenous glucocorticoids. Furthermore, the progressive increase in basal allopregnanolone and its increased response to ACTH indicate that chronic raloxifene administration exerts direct effects on the pattern of adrenal enzymes, leading to specific changes in the circulating levels of this anxiolytic progesterone metabolite. The important reduction in the circulating levels of estradiol and estrone under long-term raloxifene administration may represent a further mechanism by which this molecule may exert a protective effect against breast and endometrial malignancies.     

The brain as a target for adrenocortical steroids: cognitive implications.

It is well established that a reciprocal control exists between the brain and glucocorticoid hormones. The brain regulates adrenocortical function via hypothalamic corticotrophin releasing hormone-41 (CRH-41), glucocorticoids act at specific receptors in the hippocampus, thus promoting negative feedback mechanisms. Because the hippocampus is a major site for memory processes, a role for excessive/long-lasting plasma glucocorticoid levels has been suggested in conditions of mental impairment. Major depression, Cushing's disease, and dementia of the Alzheimer type are disorders which share hyperactivity of the hypothalamo-pituitary-adrenal axis, as well as symptoms of cognitive decline. Although the mechanisms leading to hypercortisolemia appear to be different in each case, the neuropsychological features of these three disorders accord with the hypothesis of glucocorticoid-associated brain damage. It therefore is important to find pharmacological strategies that will avert or reduce these potential consequences on brain function.     

Immunohistochemical study of copper-zinc and manganese superoxide dismutases in the lungs of human fetuses and newborn infants: Developmental profile and alterations in hyaline membrane disease and bronchopulmonary dysplasia

To determine the late gestational development of copper-zinc (CnZn) and manganese (Mn) superoxide dismutases (SOD) in human lung, immunohistochemical localization was performed for each SOD. The lung samples were taken from five aborted fetuses, four fetuses in which intrauterine death occurred, one full-term neonate, two premature infants with hyaline membrane disease and one premature infant with bronchopulmonary dysplasia (BPD). Morphometry was performed, and the percent area of positive staining was computed. The bronchial epithelium was intensely stained from the early stages of gestation (i.e. 17 weeks), while the staining intensity for both CuZnSOD and MnSOD in the peripheral airways increased gradually during lung development. The mean percent area of the staining for CuZnSOD and MnSOD from 16 to 38 weeks was increased 30-fold and 8-fold, respectively, and further increases were observed postnatally. CuZnSOD staining was markedly decreased in lungs with respiratory disorders. However, proliferating type II pneumocytes were intensely stained for MnSOD in the BPD lungs, making the staining area 3-fold larger than that in the control lungs. These results clearly depict age-related increases in staining for both CuZnSOD and MnSOD and an alteration in SOD distribution associated with neonatal respiratory disorders.     

Cotrimoxazole therapy ofToxoplasma gondii encephalitis in AIDS patients

Twenty-four consecutive HIV-positive patients affected byToxoplasma gondii encephalitis received trimethoprim-sulfamethoxazole (cotrimoxazole) as acute-phase treatment. Two dosage regimens of cotrimoxazole were used: 40 mg/kg/day (12 patients) or 120 mg/kg/day (12 patients) of total compound (trimethoprim plus sulfamethoxazole). Clinical and radiological responses to treatment were evaluated, and the product-limit method for survival data analysis was used. Eighteen of 24 patients showed both a clinical and radiological response (75 % response rate). There were no differences in response rates between patients receiving the two dosage regimens of cotrimoxazole. Adverse reaction consisted of leukopenia (two cases) and skin rash (three cases) which led to the discontinuation of the drug in one case. These results suggest that a randomized, controlled clinical trial should be carried out comparing cotrimoxazole versus sulfadiazine-pyrimethamine in AIDS patients withToxoplasma gondii encephalitis.Deceased.